Hydroa vacciniforme-like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in Caucasians.

Patients with classic hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) typically have high levels of Epstein-Barr virus (EBV) DNA in T cells and/or NK cells in blood, and skin lesions induced by sun exposure that are infiltrated with EBV-infected lymphocytes. HVLPD is very rare in the United States and Europe, but more common in Asia and South America. The disease can progress to a systemic form which may result in fatal lymphoma. We report our 11-year experience with 16 HVLPD patients from the United States and England and found that Caucasians were less likely to develop systemic EBV disease (1/10) than non-Caucasians (5/6). All (10/10) of the Caucasians were generally in good health at last follow-up, while two-thirds (4/6) of the non-Caucasians required hematopoietic stem cell transplantation. Non-Caucasians had later age of onset of HVLPD than Caucasians (median age 8 vs. 5 years), higher levels of EBV DNA (median 1,515,000 vs. 250,000 copies/ml), and more often had low numbers of NK cells (83% vs. 50% of patients) and T cell clones in the blood (83% vs. 30% of patients). RNA-seq analysis of an HVLPD skin lesion in a Caucasian compared with his normal skin showed increased expression of IFN-γ and chemokines that attract T cells and NK cells. Thus, Caucasian patients with HVLPD were less likely to have systemic disease with EBV and had a much better prognosis than non-Caucasians. clinicaltrials.gov ID: NCT00369421, NCT00032513.